Secondary Polycythemia

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Background

The word polycythemia indicates increased red blood cells, white blood cells, and platelets. Most of the time, it is used in place of erythrocythemia, or pure red blood cell increase, such as in secondary polycythemia.

The term polycythemia is reserved for the myeloproliferative disorder called polycythemia vera, in which all 3 peripheral blood cell lines can be increased.[1, 2]

Erythrocytosis or erythrocythemia is a more specific term that is used to denote increased red blood cells.[3]

Pathophysiology

Increased hemoglobin and hematocrit values reflect the ratio of red blood cell mass to plasma volume. Any change in either the hemoglobin or the hematocrit can alter test results.

Relative polycythemia, or erythrocythemia, results from decreased plasma volume (G a isb ö ck syndrome). A true polycythemia or erythrocythemia results from increased red blood cell mass. Therefore, hemoglobin and hematocrit levels cannot accurately help make this distinction. Direct measurement of red blood cell mass is necessary to differentiate these conditions.

In primary polycythemia, the disorder results from a mutation expressed within the hematopoietic stem cell or progenitor cells, which drives the eventual accumulation of red blood cells. The secondary polycythemic disorders may be acquired or congenital; however, they are driven by circulating factors that are independent of the function of hematopoietic stem cells.

Epidemiology

Frequency

United States

The frequency of secondary polycythemia depends on the underlying disease.

Mortality/Morbidity

The mortality and morbidity of secondary polycythemia depend on the underlying condition.

History

Physical

Causes

Secondary polycythemia is defined as an absolute increase in red blood cell mass that is caused by enhanced stimulation of red blood cell production. In contrast, polycythemia vera is characterized by bone marrow with an inherent increased proliferative activity.[1, 3, 5, 6, 7, 8, 9, 10] Approximately two thirds of patients with polycythemia vera have elevated white blood cell (granulocyte, not lymphocyte) counts and platelet counts.[11] No other causes of polycythemia/erythrocytosis are associated with elevated granulocyte or platelet counts.

Enhanced erythroid stimulation results from the following:

Laboratory Studies

Imaging Studies

Histologic Findings

Increased total red blood cell mass determines true polycythemia. Secondary causes must be identified individually.

Medical Care

The development of secondary erythrocytosis in response to tissue hypoxia is physiologic and probably beneficial to many patients. The expanded red blood cell mass may partially or totally compensate for the lack of oxygen delivery and result in tissue oxygenation to its normal level. However, limitation of compensatory increased red blood cells compromises circulation because of hyperviscosity when the hematocrit reaches levels higher than 60-65%. The latter leads to greater tissue hypoxia and EPO secretion, a continued increase in red blood cells, and further impairment of circulation.

Surgical Care

Some cases of secondary polycythemia are caused by conditions that can be ameliorated by surgical removal or correction.

Medication Summary

No medications are available to treat the blood disorder of secondary polycythemia. Treat the underlying health problem.

Complications

Prognosis

Author

Srikanth Nagalla, MBBS, MS, Director, Clinical Hematology, Cardeza Foundation for Hematologic Research; Assistant Professor of Medicine, Division of Hematology, Associate Program Director, Hematology/Medical Oncology Fellowship, Assistant Program Director, Internal Medicine Residency, Jefferson Medical College of Thomas Jefferson University

Disclosure: Nothing to disclose.

Coauthor(s)

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Disclosure: Nothing to disclose.

Specialty Editors

Karen Seiter, MD, Professor, Department of Internal Medicine, Division of Oncology/Hematology, New York Medical College

Disclosure: Novartis Honoraria Speaking and teaching; Novartis Consulting fee Speaking and teaching; Ariad Honoraria Speaking and teaching; Celgene Honoraria Speaking and teaching

Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Ronald A Sacher, MB, BCh, MD, FRCPC, Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Disclosure: Glaxo Smith Kline Honoraria Speaking and teaching

Rajalaxmi McKenna, MD, FACP, Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Disclosure: Nothing to disclose.

Chief Editor

Koyamangalath Krishnan, MD, FRCP, FACP, Paul Dishner Endowed Chair of Excellence in Medicine, Professor of Medicine and Chief of Hematology-Oncology, James H Quillen College of Medicine at East Tennessee State University

Disclosure: Nothing to disclose.

References

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